Irritability, dystonia, poor feeding, burnt sugar smell in diaper. Dx? Enzyme deficient? Accumulation of?
a) Maple syrup urine disease; Branched-chain α-ketoacid dehydrogenase; Branched-chain amino acids
b) Phenylketonuria; Phenylalanine hydroxylase; Phenylalanine
c) Homocystinuria; Cystathionine synthase; Methionine
d) Glycogen storage disease type I; Glucose-6-phosphatase; Glucose